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Blood-sugar levels naturally fluctuate throughout the day, the body normally maintaining levels between 70 and 110 mg/dL (3.9–6.1 mmol/L). [ 3 ] [ 2 ] Although 70 mg/dL (3.9 mmol/L) is the lower limit of normal glucose, symptoms of hypoglycemia usually do not occur until blood sugar has fallen to 55 mg/dL (3.0 mmol/L) or lower.
Symptoms of diabetic hypoglycemia, when they occur, are those of hypoglycemia: neuroglycopenic, adrenergic (that is, activating adrenergic receptors, resulting e.g. in fast heartbeat), and abdominal. Symptoms and effects can be mild, moderate or severe, depending on how low the glucose falls and a variety of other factors.
Glycogen storage disease type I (GSD I) is an inherited disease that prevents the liver from properly breaking down stored glycogen, which is necessary to maintain adequate blood sugar levels. GSD I is divided into two main types, GSD Ia and GSD Ib, which differ in cause, presentation, and treatment.
A few miscellaneous symptoms are harder to attribute to either of these causes. In most cases, all effects are reversed when normal glucose levels are restored. There are uncommon cases of more persistent harm, and rarely even death due to severe hypoglycemia of this type.
Glycogen storage disease type VI (GSD VI) is a type of glycogen storage disease caused by a deficiency in liver glycogen phosphorylase or other components of the associated phosphorylase cascade system. [2] It is also known as "Hers' disease", after Henri G. Hers, who characterized it in 1959. [3]
As glycogen stores start to be depleted, the liver begins oxidizing fatty acids to ultimately yield ketone bodies, which can serve as an alternative fuel source for the brain in the absence of glucose. [3] Therefore, the combination of low glucose (hypoglycemia) and the presence of ketone bodies yields the state known as ketotic hypoglycemia.
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